Advance Care Planning in Adults with Congenital Heart Disease: A Patient Priority.

Background: Adult congenital heart disease (ACHD) patients with moderate or great defect complexity are at risk for premature death. Although early engagement in advance care planning (ACP) is recommended, previous research suggests that it seldom occurs.

Methods: This study investigated ACHD patient preferences for ACP and factors that impact preferences.

The Retroauricular Approach to the Facial Nerve Trunk.

Background: Exposure of the common trunk of the facial nerve has traditionally been approached based on principles of parotidectomy, which is associated with high rates of facial nerve palsy and landmarks that may be unreliable. On the basis of experience gained with vascularized composite allotransplantation of the face, the authors propose a retroauricular approach that may be more time-effective and safe.

Methods: In the proposed retroauricular facial nerve approach, an incision is made posterior to the ear in the retroauricular sulcus, and dissection proceeds anteriorly to the mastoid fascia to the base of the conchal bowl.

Readmissions after adult congenital heart surgery: Frequency and risk factors.

Objective: Despite their clinical importance, 30-day readmission after adult congenital heart surgery has been understudied. They sought to determine the frequency of unplanned readmissions after adult congenital heart surgery and to identify any potential associated risk factors.

Design: Retrospective cohort study using State Inpatient Databases for Washington, New York, Florida, and California from 2009 to 2011.

A Novel Nonlinear Mathematical Model of Thoracic Wall Mechanics During Cardiopulmonary Resuscitation Based on a Porcine Model of Cardiac Arrest.

Cardiopulmonary resuscitation (CPR) is used widely to rescue cardiac arrest patients, yet some physiological aspects of the procedure remain poorly understood. We conducted this study to characterize the dynamic mechanical properties of the thorax during CPR in a swine model. This is an important step toward determining optimal CPR chest compression mechanics with the goals of improving the fidelity of CPR simulation manikins and ideally chest compression delivery in real-life resuscitations.

The timing and prevalence of intraoperative hypotension in infants undergoing laparoscopic pyloromyotomy at a tertiary pediatric hospital.

Background: Intraoperative hypotension may be associated with adverse outcomes in children undergoing surgery. Infants and neonates under 6 months of age have less autoregulatory cerebral reserve than older infants, yet little information exists regarding when and how often intraoperative hypotension occurs in infants.

Aims: To better understand the epidemiology of intraoperative hypotension in infants, we aimed to determine the prevalence of intraoperative hypotension in a generally uniform population of infants undergoing laparoscopic pyloromyotomy.

An Unusual Lacerated Tracheal Tube during Le Fort Surgery: Literature Review and Case Report.

Maxillofacial surgeries can present unique anesthetic challenges due to potentially complex anatomy and the close proximity of the patient's airway to the surgical field. Damage to the tracheal tube (TT) during maxillofacial surgery may lead to significant airway compromise. We report the management of a patient with a partially severed TT during Le Fort surgery for midfacial hypoplasia and management strategies based on peer-reviewed literature.

An Intrinsically Disordered Region of the DNA Repair Protein Nbs1 Is a Species-Specific Barrier to Herpes Simplex Virus 1 in Primates.

Humans occasionally transmit herpes simplex virus 1 (HSV-1) to captive primates, who reciprocally harbor alphaherpesviruses poised for zoonotic transmission to humans. To understand the basis for the species-specific restriction of HSV-1 in primates, we simulated what might happen during the cross-species transmission of HSV-1 and found that the DNA repair protein Nbs1 from only some primate species is able to promote HSV-1 infection. The Nbs1 homologs that promote HSV-1 infection also interact with the HSV-1 ICP0 protein.

Clinical Decision Support Tool for Parental Tobacco Treatment in Hospitalized Children.

Objectives: To create and evaluate the feasibility, acceptability, and usability of a clinical decision support (CDS) tool within the electronic health record (EHR) to help pediatricians provide smoking cessation counseling and treatment to parents of hospitalized children exposed to secondhand smoke (SHS).

Methods: Mixed method study of first-year pediatric residents on one inpatient unit. Residents received training in smoking cessation counseling, nicotine replacement therapy (NRT) prescribing, and use of a CDS tool to aid in this process.

Skeletal muscle and plasma concentrations of cefazolin during complex paediatric spinal surgery.

Background: Surgical site infections (SSIs) can have devastating consequences for children who undergo spinal instrumentation. Prospective evaluations of prophylactic cefazolin in this population are limited. The purpose of this study was to describe the pharmacokinetics and skeletal muscle disposition of prophylactic cefazolin in a paediatric population undergoing complex spinal surgery.

Parent and Adolescent Interest in Receiving Adolescent Health Communication Information From Primary Care Clinicians.

Purpose: Patient-centered health care recognizes that adolescents and parents are stakeholders in adolescent health. We investigate adolescent and parent interest in receiving information about health topics and parent-teen communication from clinicians.

Methods: Ninety-one parent-adolescent dyads in one practice completed individual interviews.

Missed Newborn Screening Case of Carnitine Palmitoyltransferase-II Deficiency.

Carnitine palmitoyltransferase-II (CPT-II) deficiency can be detected through newborn screening with tandem mass spectrometry. We report a 4-year-old patient with rhabdomyolysis due to CPT-II deficiency, which was initially missed by newborn screening. The patient presented with a 2-day history of fevers, upper respiratory infection, diffuse myalgia, and tea-colored urine.

Utility of neurocognitive testing of mild traumatic brain injury in children treated and released from the emergency department.

Primary Objective: To assess feasibility and utility of neurocognitive testing of children evaluated and discharged from the ED with mild traumatic brain injury (MTBI).

Methods: Paediatric blunt trauma patients (aged 11-18 years) evaluated in the ED for MTBI and control patients with isolated lower extremity injury were prospectively enrolled. All patients were administered a validated neurocognitive test (ImPACT(©)).

Neural crest requires Impdh2 for development of the enteric nervous system, great vessels, and craniofacial skeleton.

Mutations that impair the proliferation of enteric neural crest-derived cells (ENCDC) cause Hirschsprung disease, a potentially lethal birth defect where the enteric nervous system (ENS) is absent from distal bowel. Inosine 5' monophosphate dehydrogenase (IMPDH) activity is essential for de novo GMP synthesis, and chemical inhibition of IMPDH induces Hirschsprung disease-like pathology in mouse models by reducing ENCDC proliferation. Two IMPDH isoforms are ubiquitously expressed in the embryo, but only IMPDH2 is required for life.

Posttraumatic stress in school-age children and adolescents: medical providers' role from diagnosis to optimal management.

Millions of children and adolescents each year are exposed to potentially traumatic events (PTEs), placing them at risk for posttraumatic stress (PTS) disorder symptoms. Medical providers play an important role in the identification and treatment of PTS, as they are typically the initial point of contact for families in the wake of a PTE or during a PTE if it is medically related (eg, injury/illness). This paper offers a review of the literature focused on clinical characteristics of PTS, the assessment and diagnosis of PTS, and current effective treatments for PTS in school-age children and adolescents.

Concept and design of a genome-wide association genotyping array tailored for transplantation-specific studies.

Background: In addition to HLA genetic incompatibility, non-HLA difference between donor and recipients of transplantation leading to allograft rejection are now becoming evident. We aimed to create a unique genome-wide platform to facilitate genomic research studies in transplant-related studies. We designed a genome-wide genotyping tool based on the most recent human genomic reference datasets, and included customization for known and potentially relevant metabolic and pharmacological loci relevant to transplantation.

Hepatocyte Growth Factor and MET Support Mouse Enteric Nervous System Development, the Peristaltic Response, and Intestinal Epithelial Proliferation in Response to Injury.

Unlabelled: Factors providing trophic support to diverse enteric neuron subtypes remain poorly understood. We tested the hypothesis that hepatocyte growth factor (HGF) and the HGF receptor MET might support some types of enteric neurons. HGF and MET are expressed in fetal and adult enteric nervous system.

Perioperative Smartphone Apps and Devices for Patient-Centered Care.

Smartphones have grown in ubiquity and computing power, and they play an ever-increasing role in patient-centered health care. The "medicalized smartphone" not only enables web-based access to patient health resources, but also can run patient-oriented software applications and be connected to health-related peripheral devices. A variety of patient-oriented smartphone apps and devices are available for use to facilitate patient-centered care throughout the continuum of perioperative care.

Predictors of arthritis in pediatric patients with lupus.

Background: Arthritis is one of the most common manifestations of systemic lupus erythematosus (SLE). Although typically non-erosive and non-deforming, children with SLE arthritis can have significant morbidity with decreased quality of life. Our goal was to identify potential clinical and laboratory predictors of arthritis in a cohort of pediatric patients with SLE.

The impact of parental history on children's risk of asthma: a study based on the National Health and Nutrition Examination Survey-III.

Purpose: This study aimed to examine the separate effects of maternal and paternal history on the onset of asthma in children and evaluate the relationship between age of asthma onset in parents and risk of asthma in their children.

Methods: We used data from the third National Health and Nutrition Examination Survey. We developed new continuous standardized scores for survey data to quantify parental history that incorporated both the occurrence of asthma and the age at onset, and associated these scores with asthma risk in the children.

Retention rates of rufinamide in pediatric epilepsy patients with and without Lennox-Gastaut Syndrome.

Objective: To evaluate the effectiveness of rufinamide (RFM) in patients with Lennox-Gastaut Syndrome (LGS) compared to those with other epilepsy syndromes using time to treatment failure (retention rate) as the outcome measure.

Methods: In this retrospective cohort study, characteristics and outcomes of all patients receiving RFM in 2009 and 2010 were recorded. The primary outcome measure was RFM failure, defined as discontinuation of RFM or initiation of an additional antiepileptic therapy.

Role of astrocytes in epilepsy.

Astrocytes express ion channels, transmitter receptors, and transporters and, thus, are endowed with the machinery to sense and respond to neuronal activity. Recent studies have implicated that astrocytes play important roles in physiology, but these cells also emerge as crucial actors in epilepsy. Astrocytes are abundantly coupled through gap junctions allowing them to redistribute elevated K(+) and transmitter concentrations from sites of enhanced neuronal activity.

Predictors of kidney disease in a cohort of pediatric patients with lupus.

Introduction: Children with systemic lupus erythematosus (SLE) have an increased prevalence of kidney disease compared to their adult counterparts. Our goal was to identify potential clinical and laboratory predictors of renal disease.

Methods: We performed a cohort study of incident and prevalent patients with SLE aged ≤19 years.

Big data and visual analytics in anaesthesia and health care.

Advances in computer technology, patient monitoring systems, and electronic health record systems have enabled rapid accumulation of patient data in electronic form (i.e. big data).